Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that features the formation of scar tissue between the walls of the lungs’ air sacs. As this scar tissue thickens and stiffens, the lungs aren’t able to take in oxygen as efficiently.

An acute exacerbation of IPF is a relatively sudden, unexplained worsening of the condition. Basically, the scarring in a person’s lungs becomes much worse, and the person develops extreme difficulty breathing. This shortness or loss of breath is even worse than before.

A person with exacerbations may have medical conditions, such as an infection or heart failure. However, these other conditions won’t be severe enough to explain their extreme breathing problems.

Unlike exacerbations in other lung diseases such as chronic obstructive pulmonary disease (COPD), in IPF it’s not simply a matter of having extra trouble breathing. The damage caused by IPF is permanent. The term “acute” simply means that the deterioration happens rather quickly, typically within 30 days.

Without understanding the risk factors, knowing if you’ll have an acute exacerbation is difficult to predict. Researchers don’t necessarily agree on the rates of acute exacerbations.

One study determined that about 14 percent of people with IPF would experience an acute exacerbation within a year of diagnosis and about 21 percent within three years. In clinical trials, the incidence seems to be much lower.

the removal of certain immune system cells to see how this may slow the progression of IPF or reduce the risk of acute exacerbation

While it’s much too soon to know whether any of this research will result in an effective treatment for acute exacerbations, it’s encouraging to know that more attention is being paid to this relatively unknown condition. Learn more about the future of IPF treatment here.

Medically reviewed by Alana Biggers, MD on June 15, 2018 — Written by Stephanie Pierce